An inherited lifelong bleeding disorder similar to haemophilia
In von Willebrand’s disease, a substance called von Willebrand factor is absent from the blood or is present only in very low levels. Von Willebrand factor binds to a blood protein called Factor VIII, which is needed for blood clotting. A deficiency may lead to excessive or prolonged bleeding.
Von Willebrand’s disease is a rare disorder caused by a dominant gene (see Gene disorders); a person needs to inherit the gene from only one parent to develop the condition.
The symptoms of von Willebrand’s disease include easy bruising, frequent nosebleeds, bleeding gums, heavy menstrual periods in women, and prolonged bleeding from even minor cuts.
A diagnosis is usually made by determining how long your blood takes to clot and measuring the levels of clotting factors in your blood.
In mild cases, treatment is not usually necessary. If you bleed often or are due to have major surgery, you may be prescribed the drug desmopressin (see Pituitary drugs), which raises the levels of von Willebrand factor and Factor VIII in your blood. Women may be advised to take an oral contraceptive to prevent heavy menstrual periods. If bleeding is severe, a blood extract rich in clotting factors may be given intravenously. The condition is usually mild, and, with treatment, the outlook is good.
From the 2010 revision of the Complete Home Medical Guide © Dorling Kindersley Limited.